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[Reye's syndrome: pathogenetic problems (author's transl)]

G Sabetta, M Castro, A Donfrancesco

    La Pediatria Medica E Chirurgica : Medical and Surgical Pediatrics
    |November 1, 1981
    PubMed
    Summary
    This summary is machine-generated.

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    Reye

    Area of Science:

    • Biochemistry
    • Pediatrics
    • Neurology

    Context:

    • Reye's syndrome is a rare but serious condition affecting children and teenagers.
    • It is characterized by swelling in the brain and fatty liver.
    • The exact cause is often unknown, but it is associated with viral infections and aspirin use.

    Purpose:

    • To investigate the role of urea cycle enzyme deficiencies in Reye's syndrome.
    • To explore potential toxic and metabolic factors contributing to the condition.

    Summary:

    • This study describes six fatal cases of Reye's syndrome despite treatment with exchange-transfusions and infusions of citrulline and ornithine.
    • Autopsies revealed cerebral edema and fatty liver degeneration.
    • Enzyme analysis showed absent or normal ornithine transcarbamylase (OTC) activity in three patients, and partial carbamoyl phosphate synthetase (CPS) deficiency in one.

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  • The findings suggest Reye's syndrome may involve factors beyond urea cycle enzyme deficiencies, including toxic and metabolic causes.

    • Impact:

    • Highlights the complex etiology of Reye's syndrome, extending beyond known urea cycle disorders.
    • Underscores the need for further research into the multifactorial nature of Reye's syndrome.
    • Informs clinical understanding and potential diagnostic approaches for Reye's syndrome.